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Aortic root dilation in adult patients with Marfan syndrome: Does aortic root stiffness matter?

Kelly Cox1, Yousi A Oquendo2, David Liang3

  • 1Division of Cardiology, Department of Pediatrics, Lucile Packard Children's Hospital, Stanford University, Palo Alto, Calif.

JTCVS Open
|August 25, 2022
PubMed
Abstract

Insights

Aortic stiffness predicts aortic dilation rate in Marfan syndrome (MFS) patients, unlike aortic root size. This finding may improve clinical management strategies for MFS vascular complications.

Area of Science:

  • Cardiology
  • Vascular Biology
  • Genetics

Background:

  • Aortic root (AoR) size is an imperfect predictor of aortic dilation in Marfan syndrome (MFS).
  • Aortic stiffness is a potential indicator of vascular phenotype and predictor of aortic dilation.
  • Assessing AoR dilation rate and stiffness in adult MFS patients is crucial for understanding disease progression.

Purpose of the Study:

  • To assess the rate of AoR dilation and stiffness in adult patients with MFS.
  • To determine if AoR stiffness is a better predictor of AoR dilation rate than AoR size in MFS.
  • To identify factors associated with AoR dilation and the need for aortic surgery in MFS.

Main Methods:

  • Retrospective chart review of adult MFS patients (aged 20-40 years) with at least two echocardiograms 6 months apart.
  • Calculation of AoR dilation rate and stiffness using echocardiographic data analyzed by a blinded observer.
  • Comparison of baseline characteristics, AoR dimensions, stiffness, and dilation rates between patients who underwent aortic surgery (Sx) and those who did not (NSx).

Main Results:

  • Baseline AoR stiffness, but not baseline age or AoR dimension, was significantly associated with AoR dilation rate (P < .001).
  • Patients who eventually required AoR surgery had significantly larger baseline AoR dimensions (P = .011) and higher AoR stiffness (P = .024) compared to those who did not.
  • AoR dilation rate was significantly greater in patients who underwent surgery, independent of baseline AoR dimension (P = .01).

Conclusions:

  • AoR dilation rate in adult MFS patients varies and is associated with baseline AoR stiffness measured by echocardiography.
  • Aortic stiffness may serve as a more accurate predictor of aortic dilation progression in MFS than AoR size alone.
  • Further research is needed to integrate aortic stiffness measurements into clinical practice for refined MFS management.
Keywords:
AoR, aortic rootEM, elastic modulusMFS, Marfan syndromeMarfan syndromeNSx, no surgerySI, stiffness indexSx, Surgeryaortic rootdilationstiffness

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